Abstract:
:The copper concentrations in organs of developing Long-Evans Cinnamon (LEC) rats (2 d to 13 mo) were measured to elucidate the pathogenesis of their hereditary hepatitis. Hepatic copper contents of LEC rats were significantly higher than those of control rats (26 to 92 times higher). The subcellular distribution of hepatic copper indicated that the nuclear and large granular fractions had been saturated and the cytosol fraction contained about 70% of all the hepatic copper in LEC rats. The serum concentrations of copper and ceruloplasmin were significantly lower than those of control rats from the 4th wk (10-12% and 5-19%, respectively). Copper contents in kidney of LEC rats did not exhibit an increase over those of control rats until 12 wk, but then increased to nearly 40 times higher during fulminant hepatic failure. Accumulation of copper was not detected in the brain or small intestines of LEC rats until 13 mo. The hepatic copper concentration, its subcellular distribution, and serum copper concentration of F1 rats (LEC x Long-Evans Agouti) exhibited the same levels as those of Long-Evans Agouti rats. In addition to their similarity concerning inheritance of autosomal recessive means and clinical course, we found causality relating copper accumulation to the pathogenesis of the disease. We propose that LEC rats will be the most promising animal model for the study of Wilson's disease.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Okayasu T,Tochimaru H,Hyuga T,Takahashi T,Takekoshi Y,Li Y,Togashi Y,Takeichi N,Kasai N,Arashima Sdoi
10.1203/00006450-199203000-00011subject
Has Abstractpub_date
1992-03-01 00:00:00pages
253-7issue
3eissn
0031-3998issn
1530-0447journal_volume
31pub_type
杂志文章abstract::DNA segments that are separated from the promoter region of a gene by many thousands of bases may nonetheless regulate the transcriptional activity of that gene. This finding has led to the investigation of mechanisms underlying long-range chromatin interactions. In intermitotic cells, chromosomes decondense, filling ...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/pdr.0b013e31804575db
更新日期:2007-05-01 00:00:00
abstract::Plasma selenium concentration and glutathione peroxidase activity in red blood cells were determined in subjects from different age groups. The selenium level (mean +/- SD) found in infancy (0 to 6 months) was 2 +/- 0.6 micrograms/dl, with the lowest value of 1 microgram/dl observed in a 4-month-old infant. These leve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00021
更新日期:1986-06-01 00:00:00
abstract:BACKGROUND:Toll-like receptor (TLR)-mediated inflammation may contribute to neonatal sepsis, for which pentoxifylline (PTX), a phosphodiesterase inhibitor that raises intracellular cAMP, is a candidate adjunctive therapy. We characterized the anti-inflammatory effects of PTX toward TLR-mediated production of inflammato...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.6
更新日期:2017-05-01 00:00:00
abstract::The purpose of this investigation was twofold: (1) to identify and characterize the enzymatic ATP hydrolysis system of epithelial cilia, and (2) to develop a quantitative, biochemical test for the ciliotoxic cystic fibrosis (CF) factor based on inhibition of ATP utilization by ciliary preparations. Our rationale for s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197602000-00012
更新日期:1976-02-01 00:00:00
abstract::We probed possible developmentally related changes in thin filament activity in rat hearts with the aid of calmidazolium (CDZ). CDZ is a calmodulin antagonist that also binds to troponin C and stimulates Ca2+ troponin C-dependent activation of cardiac myofibrillar contractile activity. In paired experiments, we compar...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199007000-00011
更新日期:1990-07-01 00:00:00
abstract::Defective arousal mechanisms are viewed as contributory to sleep hypopnea disorders and sudden infant death syndrome. Sighs (i.e. augmented breaths) as well as startles have not traditionally been viewed as arousal-related phenomena in infants. We hypothesized that, if sighs and startles are the initial event in a seq...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199811000-00021
更新日期:1998-11-01 00:00:00
abstract::We hypothesized that fetal pancreatic digestive enzymes play a role in the lung damage after meconium aspiration. We studied the effect of meconium on the A549 alveolar epithelial cell line. The exposure of the cells to 0.5 to 5% meconium resulted in significant disruption of connections between A549 cells and caused ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181ebd4c3
更新日期:2010-09-01 00:00:00
abstract::During the last few years, advances in the care of low-birth-weight and preterm neonates has stimulated research on the best dietetic program to improve survival and to reduce handicap incidence. At present, fortification of human milk with artificial formulas is the most usual dietetic solution. As yet, however, litt...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199905010-00008
更新日期:1999-05-01 00:00:00
abstract::The odds risk of vertical transmission of human immunodeficiency virus (HIV) to preterm infants is almost four times that of term infants and may relate to maternal and neonatal factors. We characterized the competence of early nonspecific cellular immunity, namely natural killer cytotoxicity (NKC) and antibody-depend...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199609000-00021
更新日期:1996-09-01 00:00:00
abstract::Experiments were done to investigate the effects of increased inspired oxygen on the arousal response from sleep to upper airway obstruction in 10 newborn lambs. Each lamb was anesthetized and instrumented for recordings of electrocorticograms, electrooculogram, nuchal and diaphragm electromyograms, and measurements o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198702000-00002
更新日期:1987-02-01 00:00:00
abstract:BACKGROUND:Kawasaki disease (KD) is associated with expression and methylation of Fc gamma receptor genes. We characterized immunoglobulin A (IgA), IgE, IgG, and IgM receptor expression levels in KD. METHODS:Fc receptor expression levels were characterized using GeneChip Human Transcriptome Array 2.0 (HTA 2.0) with 18...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0707-y
更新日期:2019-12-09 00:00:00
abstract::Blue and white phototherapy was given to infant (and weanling) homozygous Gunn rats treated with different doses of riboflavin-5-phosphate (ribofl.5'p.) During the first hours after a flavin-injection, the effect of phototherapy with both types of fluorescent lamps was enhanced. With equal radiant power applied, the s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197912000-00001
更新日期:1979-12-01 00:00:00
abstract::Cerebral development may be impaired in fetuses with congenital cardiovascular malformations, particularly hypoplastic left heart syndrome (HLHS) and aortopulmonary transposition (APT). The decreased cerebral arterial pusatility index observed in some of these fetuses led to the belief that cerebral vascular resistanc...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2016.65
更新日期:2016-08-01 00:00:00
abstract::Plasma aldosterone (A), corticosterone (B), deoxycorticosterone (DOC), progesterone (P), 17-hydroxyprogesterone (17-OHP), cortisol (F), and cortisone (E) were measured simultaneously by specific radioimmunoassays in small plasma samples obtained from 174 normal infants and children between 2 hr and 15 yr of age. The s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198001000-00010
更新日期:1980-01-01 00:00:00
abstract::Genetic factors cannot explain the recent rapid increase in the incidence of atopic diseases. The phenomenon has been explained by environmental factors, and there are data for and against the hypothesis that a decline in the pressure of microbial stimulation early in life could be behind the allergy epidemic. Changes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200207000-00004
更新日期:2002-07-01 00:00:00
abstract::We studied healthy and saline lavaged rabbits during high frequency oscillatory ventilation to determine what combination of frequency (f), tidal volume (Vt), and mean airway pressure (Paw) produced the lowest peak-to-peak alveolar pressure amplitude (Palv) and physiologic blood gas tensions. Sinusoidal volume changes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199001000-00018
更新日期:1990-01-01 00:00:00
abstract:BACKGROUND:The prevalence of vitamin D (vitD) deficiency presenting as rickets is increasing worldwide. Insufficient sun exposure, vitD administration, and/or calcium intake are the main causes. However, vitD system-related genes may also have a role. METHODS:Prospective study: 109 rachitic children completed a 6-mo s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.222
更新日期:2017-02-01 00:00:00
abstract::Cancer stem cells (CSC) are recently proposed to be the cancer initiating cells responsible for tumorigenesis and contribute to cancer resistance. Advances have been made in identifying and enriching CSC in leukemia and several solid tumors, including breast, brain and lung cancers. These studies suggest that, like no...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.pdr.0000203592.04530.06
更新日期:2006-04-01 00:00:00
abstract:BACKGROUND:The earliest onset of puberty had shifted downward, which may be due to the role of early growth and development factors in childhood. METHODS:All of 1575 Kindergarten Two (K2) children from Anhui province, China were followed up to elementary school. Girls (n = 342) with available data on AR and breast dev...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-1001-8
更新日期:2020-06-08 00:00:00
abstract::The proposed actions of atrial natriuretic factor (ANF) are mediated through specific plasma membrane (R1) receptors coupled to guanylate cyclase. A second receptor, R2, has been characterized by its ability to bind to an acyclic, truncated ANF analog (C-ANF4-23). The ANF-R2 receptor has not been identified in the fet...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1991-04-01 00:00:00
abstract::In its normal circulatory environment, the fetal left ventricle can maximally increase output less than 2-fold, in contrast to the nearly 3-fold increase that occurs at birth. Several studies have attributed this finding to fetal myocardial "immaturity," and speculated that there is a rapid maturation of the myocardiu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199105010-00011
更新日期:1991-05-01 00:00:00
abstract::The antiendotoxin effect of polymyxin B was investigated in experimentally induced septicemia in rabbits. The Pasteurella multocida organisms were sensitive to the antibacterial action of penicillin but not to polymyxin B. Animals pre-treated with polymyxin showed positive blood cultures and significantly reduced plas...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197901000-00011
更新日期:1979-01-01 00:00:00
abstract::Pseudohypoaldosteronism is a hereditary salt-wasting syndrome usually seen in infancy with weight loss, dehydration, and failure to thrive. The pathophysiologic origin of pseudohypoaldosteronism is unknown. The defect could be related to the unresponsiveness of target organs to mineralocorticoids resulting in hyponatr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199403000-00021
更新日期:1994-03-01 00:00:00
abstract::A girl presented with an important growth retardation, hepatomegaly, fasting hypoglycemia, lactic acidosis, increased serum cholesterol, triglycerides and uric acid, and increased liver glycogen (7.5%). There was no rise in blood glucose after IV galactose or fructose, but glucagon gave a delayed response. Type Ib gly...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198101000-00014
更新日期:1981-01-01 00:00:00
abstract::A number of neuromuscular diseases are associated with molecular defects in the mitochondrial DNA (mtDNA). These include: 1) a missense mutation at nucleotide 11778 in the mtDNA of Leber's hereditary optic neuropathy patients; 2) a heterogeneous array of deletions in the mtDNA of ocular myopathy patients; and 3) small...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199011000-00023
更新日期:1990-11-01 00:00:00
abstract::One of two slightly different influenza A/ts-1[E] recombinant candidate live vaccines was given intranasally to each of 23 young children. Twelve of 15 children who had no serum HI antibody but who did have serum ANAB at the time of administration became infected and 1 had mild rhinitis. All eight who lacked both type...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197604000-00008
更新日期:1976-04-01 00:00:00
abstract::BackgroundPregnant women at a high risk of preterm delivery receive glucocorticoids to accelerate fetal lung maturation and surfactant synthesis. However, the effect of antenatal steroids on the developing diaphragm remains unclear. We hypothesized that maternal betamethasone impairs the fetal diaphragm, and the magni...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.99
更新日期:2017-09-01 00:00:00
abstract::In artificial tubes as well as in blood vessels with diameters less than 500 microns, blood viscosity decreases with decreasing diameter (Fahraeus-Lindqvist effect). Our study measured viscosity of red blood cells (RBC) from 10 preterm infants, 10 term neonates, and 10 adults by means of a capillary viscometer. RBC we...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198906000-00009
更新日期:1989-06-01 00:00:00
abstract::Pyritinol-HCl was tested for its impact on the cognitive functions of children with learning disabilities. This study is a contribution to scientific discussion on the complicated methodologic problems in evaluating the clinical efficacy of psychopharmacologic agents. Sixty-seven pupils of slow learner classes between...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-197509000-00006
更新日期:1975-09-01 00:00:00
abstract::We have developed a method for measuring oxygen consumption (Vo2) in preterm infants in their normal incubator environment over prolonged periods. The results of measurements made over 24 h in 18 infants are presented. In normally grown infants, the mean Vo2 was 9.66 +/- 1.25 liters/kg X 24 h (SD) (6.71 +/- 0.87 ml/kg...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198410000-00003
更新日期:1984-10-01 00:00:00